Maurizi M, Ottaviani F, Paludetti G, Lungarotti S
Int J Pediatr Otorhinolaryngol. 1985 Aug;9(3):227-32. doi: 10.1016/s0165-5876(85)80038-0.
The authors have investigated the auditory function in 35 Down's subjects, aged between 1 month and 16 years. Clinical examination revealed the occurrence of impaired nasal breathing in 18 subjects (51.4%), while otoscopy results were bilaterally normal in 12 cases (34.3%). Behavioural pure-tone audiometry yielded reliable results in 10 children (28.6%), impedance tests in 28 (80.0%), and brainstem audiometry in 29 (82.9%). A clinical and audiological follow-up has been performed in 11 cases (31.4%). Pure-tone audiometry, which may be employed in all cases only beyond 8 years of age, revealed a conductive hearing loss in 7 cases (20.0%). Impedance tests, whose usefulness is limited by the high occurrence of external ear canal stenosis, showed bilateral type A tympanograms only in 8 cases (28.6%). Stapedial reflex data were often missing, even in presence of a type A tympanogram, due to the weakness of tubaric muscles and to the presence of ossicles abnormalities. ABR has been performed in 29 cases (82.9%) and resulted to be as effective as in the normal population. It revealed a normal configuration concerning threshold and morphology in 16 cases (55.1%). The authors conclude that middle ear pathology in the Down's population is more frequent than expected on clinical basis and that objective tests are mandatory in order to obtain a reliable evaluation. While impedance tests are very sensible in detecting mild middle ear pathologies, but are not effective in threshold definition, brainstem audiometry is the choice tool in the uncooperative child, even if it cannot allow a differential diagnosis between normality and mild low-frequency conductive hearing losses.
作者对35名年龄在1个月至16岁之间的唐氏综合征患者的听觉功能进行了研究。临床检查发现18名患者(51.4%)存在鼻呼吸障碍,而耳镜检查结果显示12例(34.3%)双侧正常。行为纯音听力测试在10名儿童(28.6%)中得出可靠结果,阻抗测试在28名(80.0%)患者中得出可靠结果,脑干听觉测试在29名(82.9%)患者中得出可靠结果。对11例(31.4%)患者进行了临床和听力学随访。纯音听力测试仅在8岁以上的所有病例中可用,结果显示7例(20.0%)存在传导性听力损失。阻抗测试的效用因外耳道狭窄的高发生率而受限,仅8例(28.6%)显示双侧A型鼓室图。即使存在A型鼓室图,由于咽鼓管肌肉无力和听小骨异常,镫骨肌反射数据也常常缺失。对29例(82.9%)患者进行了ABR测试,结果与正常人群一样有效。16例(55.1%)患者的阈值和形态显示正常构型。作者得出结论,唐氏综合征患者中耳病变比临床预期更为常见,客观测试对于获得可靠评估至关重要。虽然阻抗测试在检测轻度中耳病变方面非常敏感,但在阈值定义方面无效,脑干听觉测试是不合作儿童的首选工具,即使它无法区分正常与轻度低频传导性听力损失。